> The level of TSE infection in goats seems however to be extremely low> and any possible risk to consumers is minimal.

IS this assessment based on true science or Gov. PR science?

FROM what i have been told, the goat prion protein sequence is fairlydifferent from cow, so no one has any idea of what the species barrierwould be, tissue distribution infectivity, infectivity in milk orcheese, (if any) or whether it would primarilytransmit to met met or equally to val val. fact is no one really knowsyet. why do they continue to claim things with whichthey have no scientific data to back it up with, while continuing toignore other data.

goat/cow are identical at 247/256 amino acid positions, ie different at 8

Our findings show that environmental sources of infectivity maycontribute to CWD epidemics and illustrate the potential complexity ofsuch epidemics in natural populations. The relative importance ofdifferent routes of infection from the environment cannot be discernedfrom our experiment, but each could play a role in sustaining naturalepidemics. Although confinement likely exaggerated transmissionprobabilities, conditions simulated by this experiment do arise in thewild. Mule deer live in established home ranges and show strong fidelityto historic home ranges (24-26). As a result ofsuch behavior, encounters with contaminated environments will occur morefrequently than if deer movements were random. Feces and carcass remainsare routinely encountered on native ranges, thus representing naturalopportunities for exposure. Social behavior of deer, particularly theirtendency to concentrate and become sedentary on their winter range, alsomay increase the probability of coming into contact with sources ofinfection in their environment.

The ability of the CWD agent to persist in contaminated environments for>2 years may further increase the probability of transmission andprotract epidemic dynamics (8). Becauseinfectivity in contaminated paddocks could not be measured, neither theinitial levels nor degradation rate of the CWD agent in the environmentwas estimable. However, the observed persistence of the CWD agent wascomparable to that of the scrapie agent, which persisted in paddocks for?1 to 3 years after removal of naturally infected sheep (7). Similaritiesbetween the CWD and scrapie agents suggest that environmentalpersistence may be a common trait of prions. Whether persistence of theBSE prion in contaminated feed production facilities or in environmentswhere cattle reside contributed to BSE cases in the United Kingdom afterfeed bans were enacted (27) remainsuncertain but merits further consideration.

Indirect transmission and environmental persistence of prions willcomplicate efforts to control CWD and perhaps other animal priondiseases. Historically, control strategies for animal prion diseaseshave focused on infected live animals as the primary source ofinfection. Although live deer and elk represent the most plausiblemechanism for geographic spread of CWD, our data show that environmentalsources could contribute to maintaining and prolonging local epidemics,even when all infected animals are eliminated. Moreover, the efficacy ofvarious culling strategies as control measures depends in part on therates at which the CWD agent is added to and lost from the environment.Consequently, these dynamics and their implications for diseasemanagement need to be more completely understood.

Edited by D. Carleton Gajdusek, Centre National de la RechercheScientifique, Gif-sur-Yvette, France, and approved December 7, 2000(received for review October 16, 2000)

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Characterization of the CJD and Scrapie Strains. Controls were set up bytransmitting one French and one U.S. scrapie isolate from ruminants aswell as French sCJD and iCJD cases from humans. None of these revealed alesion profile or transmission characteristics similar or close to thoseof BSE or vCJD, respectively, thus extending to the present Frenchscrapie isolate the previous observation that the BSE agent wasdifferent from all known natural scrapie strains (4, 24).

The lesion profiles of sCJD and iCJD differed only slightly in severityof the lesions, but not in shape of the profile, revealing the identityof the causative agents. One of us reported the absence of similaritybetween sCJD (six cases) and U.K. scrapie (eight cases) in transmissioncharacteristics in mice (4). Herein, we madethe striking observation that the French natural scrapie strain (but notthe U.S. scrapie strain) has the same lesion profile and transmissiontimes in C57BL/6 mice as do the two human TSE strains studied. Thisstrain "affiliation" was confirmed biochemically. There is noepidemiological evidence for a link between sheep scrapie and theoccurrence of CJD in humans (25). However, such alink, if it is not a general rule, would be extremely difficult toestablish because of the very low incidence of CJD as well as theexistence of different isolates in humans and multiple strains inscrapie. Moreover, scrapie is transmissible to nonhuman primates (26). Thus, there isstill a possibility that in some instances TSE strains infecting humansdo share a common origin with scrapie, as pointed out by our findings.

Transmission of vCJD and BSE to Nonhuman Primates. vCJD transmittedreadily to the cynomolgus macaque after 2 years of incubation, which wascomparable to the transmission obtained from first-passaged macaque BSEand much shorter than the interspecies transmission of BSE. Startingwith 100 mg of BSE-macaque brain material, dilutions up to 4 µg stillprovoked disease. These data suggest that the BSE agent rapidly adaptsto primates accompanied by enhanced virulence.

Examination of macaque brain inoculated with vCJD revealed a similarpathology to that with second-passage BSE. The distribution ofvacuolation and gliosis, as well as the pattern of PrP deposition,including the dense, sometimes florid plaques, were similar to the humanvCJD and the BSE hallmarks of the first passage (1, 2). These data showthat the phenotype of BSE in primates is conserved over two passages.Moreover, they confirm that the BSE agent behaves similarly in humansand macaques, a precious finding that will prove useful in the nearfuture for the design of pathogenesis or therapeutic studies. Because ofthe number of macaques examined in this study, we can now reliably statethat the pathology, in particular the PrP deposition pattern provoked byBSE, is similar in older and very young animals. However, plaquedeposition is greater, and mature florid plaques were more numerous, inthe young, which may be correlated with a longer duration of theclinical phase observed in this animal (2). This isimportant with regard to the fact that vCJD has been diagnosed mainly inteenagers and young adults, which raises the concern that older patientsmay have been misdiagnosed because of an alternative phenotype of thedisease. One should bear in mind, however, that cynomolgus macaques areall homozygotes for methionine at codon 129 of the PrP gene. Thus, ourobservations may not be relevant to humans carrying one or both valinealleles; however, all patients with vCJD reported to date have been M/Mat this position (27).

Intravenous Transmissions to Nonhuman Primates. Brain pathology wasidentical in macaques inoculated i.c. and i.v. The i.v. route proved tobe very efficient for the transmission of BSE, as shown by the 2-yearsurvival of the animals, which is only 5 months longer than thatobtained after inoculating the same amount of agent i.c. As the i.v.injection of the infectious agent implies per se a delayed neuroinvasioncompared with a direct inoculation in the brain, this slight lengtheningof the incubation period cannot, at this stage, be interpreted as alower efficiency of infection as regards the i.c. route.

These data should be taken into account in the risk assessment ofiatrogenic vCJD transmission by i.v. administration of biologicalproducts of human origin. They also constitute an incentive for acomplete i.v. titration.Conclusions

From BSE and vCJD transmissions in nonhuman primates, a number ofconclusions can be drawn that are of major importance for human health:(i) human-adapted BSE appears to be a variant of the BSE agent that ismore virulent for humans than cattle BSE and is efficiently transmittedby the peripheral route; (ii) the detection of vCJD in unusually youngpatients is probably not because of a lack of diagnosis of cases inolder patients, thus raising the question of the source of humancontamination with BSE early in life; and (iii) iatrogenic transmissionsfrom patients with vCJD would be readily recognized by using the samediagnostic criteria as those applied to vCJD [clinical and pathologicalcriteria (27 )comprising neuronal loss and gliosis in the thalamus correlated withhigh MRI signal (28, 29)], whether suchcontaminations had occurred by the central or i.v. route. Primary andiatrogenic cases of vCJD could be distinguished on the basis of thepatient's clinical history.

The risk assessment of biological products of human origin, notablythose derived from blood, has been deeply modified by the appearance ofvCJD. We confirm that the BSE agent has contaminated humans not only inthe U.K. and the Republic of Ireland but also in France, and we showthat its pathogenic properties for primates are being enhanced by aprimary passage in humans. Considering the flow of potentiallycontaminated bovine-derived products between 1980 and 1996, it isobvious that further vCJD cases may occur outside the U.K. Thus, and inthe light of the present study, it is necessary to sustain worldwide CJDsurveillance regardless of national BSE incidence and to take allprecautionary measures to avoid iatrogenic transmissions from vCJD.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease ofsheep and goats were transmitted to squirrel monkeys (Saimirisciureus) that were exposed to the infectious agents only by theirnonforced consumption of known infectious tissues. The asymptomaticincubation period in the one monkey exposed to the virus of kuru was36 months; that in the two monkeys exposed to the virus ofCreutzfeldt-Jakob disease was 23 and 27 months, respectively; andthat in the two monkeys exposed to the virus of scrapie was 25 and32 months, respectively. Careful physical examination of the buccalcavities of all of the monkeys failed to reveal signs or orallesions. One additional monkey similarly exposed to kuru hasremained asymptomatic during the 39 months that it has been underobservation.

While familial cases of Creutzfeldt-Jakob disease are extremely rareall over the world, 3 familial clusters were observed between1983-2000 in a relatively small area situated in the North ofSlovakia. Prevalence of CJD in this area exceeded the overallprevalence in Slovakia more than 8 times. The majority of CJDpatients admitted consuming sheep brain. Most patients lived insmall secluded villages with rather common familial intermarriage.CJD affected both sexes equally. All patients were prior to thedisease mentally normal individuals. Shortly after the onset of CJDtheir mental status deteriorated remarkably with an average survivalrate of 3.6 months.

"Clusters" of CJD in Slovakia: the first laboratory evidence of scrapie.

Mitrova E, Huncaga S, Hocman G, Nyitrayova O, Tatara M.

Institute of Preventive and Clinical Medicine, Bratislava.

Epidemic-like occurrence of Creutzfeldt-Jakob disease was observed in1987 in Slovakia (Orava). Search for the cause of CJD focus indicated acoincidence of genetic and environmental risks in clustering patients.Since Spongiform Encephalopathies might be transmitted orally, (BovineSpongiform Encephalopathy), the possibility of zoonotic source of CJDcases in Orava was also considered. A deficient knowledge about theoccurrence of scrapie in Slovakia stimulated an examination of sheepwith signs of CNS disorders in two flocks of Valasky breed in Orava. Inone flock, neurohistopathological examination revealed in sheep brainslesions characteristic for scrapie. Frozen brain tissue of these animalswere used for the detection of scrapie associated fibrils. They werefound in 2 animals from the same flock. This is the first laboratoryconfirmation of scrapie in Czecho-Slovakia. The possible epidemiologicaland economical implications are emphasized.

Chronic wasting disease (CWD) is a transmissiblespongiform encephalopathy (TSE) of deer and elk,and little is known about its transmissibility to otherspecies. An important factor controllinginterspecies TSE susceptibility is prion protein (PrP)homology between the source and recipientspecies/genotypes. Furthermore, the efficiency with whichthe protease-resistant PrP (PrP-res) of onespecies induces the in vitro conversion of the normal PrP(PrP-sen) of another species to theprotease-resistant state correlates with the cross-speciestransmissibility of TSE agents. Here weshow that the CWD-associated PrP-res (PrPCWD) of cervidsreadily induces the conversion of recombinant cervid PrP-senmolecules to the protease-resistant state in accordancewith the known transmissibility of CWD between cervids. In contrast,PrPCWD-induced conversions of human and bovine PrP-sen weremuch less efficient, and conversion of ovine PrP-sen wasintermediate. These results demonstrate a barrier at themolecular level that should limit the susceptibility of these non-cervidspecies to CWD.

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Clearly, it is premature to draw firm conclusions about CWDpassing naturally into humans, cattle and sheep, but the presentresults suggest that CWD transmissions to humans would be aslimited by PrP incompatibility as transmissions of BSE or sheepscrapie to humans. Although there is no evidence that sheepscrapie has affected humans, it is likely that BSE has caused variantCJD in 74 people (definite and probable variant CJD cases todate according to the UK CJD Surveillance Unit). Given thepresumably large number of people exposed to BSE infectivity,the susceptibility of humans may still be very low compared withcattle, which would be consistent with the relatively inefficientconversion of human PrP-sen by PrPBSE. Nonetheless, sincehumans have apparently been infected by BSE, it would seem prudentto take reasonable measures to limit exposure of humans(as well as sheep and cattle) to CWD infectivity as has beenrecommended for other animal TSEs.

> ##################### Bovine Spongiform Encephalopathy> #####################>> THE TSE COMMUNITY REFERENCE LABORATORY EXPERT> GROUP ON STRAINS:> Progress report on actions from the meeting on 25th> November, 2004, Rue Froissart.> Report drafted by Marion Simmons and John Spiropoulos using data and> analysis supplied by VLA Weybridge, IAH (NPU) Edinburgh, AFSSA and> INRA.> Executive summary> These investigations have been pursued by the transfer of materials from> experimentally inoculated mice to the Veterinary Laboratories Agency> and the> Institute for Animal Health, for blind peer review. In addition,> experimental> caprine BSE was provided to AFSSA by the IAH for further Western> immunoblotting of the suspect sample alongside appropriate controls.> Codes for mouse brain groups were held by a third party (Professor> Bostock)> so that all examinations at the VLA and IAH were blinded. Results were> reported to Professor Bostock, who broke the codes and reported the> outcome to the VLA for compilation of this report. The report is> therefore> compiled by the VLA on behalf of the Expert Group, and has been agreed> with Professor Bruce (IAH).> A more comprehensive report of our findings has been provided to the> French> scientists who submitted the samples.> Conclusion> Our findings, and interpretation of the western immunoblotting work> done at> AFSSA, support the conclusion that the French caprine isolate (CH636) is> likely to contain the BSE strain.> Dr Marion M Simmons> On behalf of the EU CRL for TSE> 28th January 2005>> http://europa.eu.int/comm/food/food/biosafety/bse/crl_statement_tse_goats_28-01-05_en.pdf>>> TSS>>>> Terry S. Singeltary Sr. wrote:>>> ##################### Bovine Spongiform Encephalopathy>> #####################>>>> Référence: IP/05/105 Date: 28/01/2005>> HTML: FR>>>> EN>>>> DE>>>> EL>>>>>> PDF: FR>>>> EN>>>> DE>>>> EL>>>>>> DOC: FR>>>> EN>>>> DE>>>> EL>>>>>>>> IP/05/105>> Brussels, 28 January 2005>>>>>> Case of BSE in a goat confirmed: Commission extends testing programme>>>> A suspected case of BSE in a goat slaughtered in France in 2002 has>> been confirmed today by a panel of European scientists>> (http://europa.eu.int/comm/food/food/biosafety/bse/crl_statement_tse_goats_28-01-05_en.pdf).>>>> The European Commission proposes to step up testing to determine if>> this is an isolated incident. Although this is the first time that>> BSE has been found in a goat under natural conditions, precautionary>> measures to protect consumers from this eventuality have been applied>> in the EU for several years. The level of TSE infection in goats>> seems however to be extremely low and any possible risk to consumers>> is minimal. The European Commission asked the French authorities to>> submit their preliminary findings to the Community Reference>> Laboratory (CRL) for TSEs based in Weybridge, UK (see IP/04/1324>> ).>> TSEs are transmissible spongiform encephalopathies, namely BSE>> affecting cattle and scrapie affecting goats and sheep.>>>> Markos Kyprianou, EU Commissioner responsible for Health and Consumer>> Protection, said “I want to reassure consumers that existing safety>> measures in the EU offer a very high level of protection. This case>> was discovered thanks to the EU testing system in place in France.>> The testing programme has shown us that there is a very low incidence>> rate of TSEs in goats and allowed us to detect suspect animals so>> that they can be taken out of the food chain, as was done with this>> goat and its entire herd. I am proposing to extend testing further to>> determine whether this is an isolated incident.”>>>> Existing safety measures>>>> For many years, safety measures have been applied to all farmed>> ruminants (cattle, goats, sheep) to offer maximum public health>> protection in case BSE in goats was ever confirmed. These safety>> measures include the ban on feeding animal proteins in the form of>> meat-and-bone meal (MBM), the removal of specified risk materials>> (i.e. the removal of tissues such as brain, spinal cord, part of the>> intestines) from the food and feed chain, the slaughtering of herds>> affected by scrapie (a disease of goats and sheep similar to BSE but>> not infectious for humans), and a TSE monitoring and testing>> programme in all Member States. Over 140,000 goats have been tested>> since April 2002, including random testing of healthy animals, sick>> animals and those that die on the farm.>>>> Extension of testing regime>>>> Following this confirmation, the Commission is proposing increased>> testing for BSE among goats for at least 6 months (200 000 tests of>> healthy goats in the EU) to determine if this is an isolated>> incident. The extent of the monitoring programme will be based on the>> goat population in each Member State and will focus primarily on>> Member States where BSE is present in the cattle population. All>> confirmed TSE cases will be subjected to a three-step testing scheme,>> already in use, which will make it possible to differentiate between>> scrapie and BSE. These additional measures will be submitted for>> Member States approval at the next meeting of the Standing Committee>> on the Food Chain and Animal Health scheduled on 2-3 February 2005..>>>> Does this BSE case indicate a widespread problem?>>>> The conditions that existed when the affected goat was born in 2000>> no longer exist and available evidence would suggest that even if the>> infection still exists in goats, the level would be extremely low.>> The feeding of meat-and-bone meal (MBM) to ruminants is generally>> considered to be the transmission route of BSE. In January 2001 the>> existing ban on feeding MBM to all ruminants was extended to a total>> ban on feeding MBM to all farmed animals. Goats in the EU generally>> only live for a few years, which means that the majority of goats in>> the EU today were born after the total feed ban was put in place.>>>> Are goat milk, cheese and meat safe?>>>> The European Food Safety Authority has advised that based on current>> scientific knowledge, goat milk and derived products are unlikely to>> present any risk of TSE contamination if the milk comes from healthy>> animals:>> http://www.efsa.eu.int/science/biohaz/biohaz_documents/709/bdoc_statement_goatsmilk_en1.pdf>>>>>> Currently, as a precautionary measure and following scientific>> advice, milk and meat from goats which are affected by TSE cannot be>> used. These rules were in place before the case of BSE in a goat was>> discovered. As for cattle and sheep, specified risk materials (the>> tissues most likely to carry infectivity if the disease is present)>> are also removed from all goats even if there is no infection>> detected. While it is not possible to say that there is absolutely no>> risk, any potential risk will be mitigated by the safety measures put>> in place.>>>> In light of the above, the European Commission advises no change in>> current consumption of goat milk, cheese and meat. The European>> Commission has asked EFSA to carry out a quantitative risk assessment>> for goat meat and goat meat products, which is expected to be ready>> by July 2005.>>>> Background>>>> Following the findings by a research group in France of a suspected>> BSE infection in a goat, the European Commission immediately made the>> findings public on 28 October 2004 (see IP/04/1324>> ).>> The supporting data were submitted on 5 November, as foreseen by the>> EU procedure, by the French authorities to the Community Reference>> Laboratory (CRL) for TSEs based in Weybridge (UK), for an evaluation>> by an expert panel. The CRL expert panel reported their findings>> today>> (http://europa.eu.int/comm/food/food/biosafety/bse/crl_statement_tse_goats_28-01-05_en.pdf).>>>>>> The infected goat was born in March 2000 and slaughtered in France in>> October 2002. The results are only now becoming available as the>> series of confirmatory tests included testing on mice (a so-called>> “mouse bioassay”), which takes two years to complete.>>>> The goat and its herd were disposed in accordance with EU rules and>> did not enter either the food or feed chain, and therefore do not>> represent a risk to public health. This goat was detected as part of>> the EU wide surveillance programme designed to detect suspicious TSE>> strains in small ruminants, and was the only one in its herd of 300>> goats to develop BSE. Over 140,000 goats have been tested across>> Europe since April 2002.>>>> See also MEMO/05/29>> .>>>>>> http://europa.eu.int/rapid/pressReleasesAction.do?reference=IP/05/105&format=HTML&aged=0&language=EN&guiLanguage=fr>>>>>> TSS>>>> ######### https://listserv.kaliv.uni-karlsruhe.de/warc/bse-l.html>> ##########>>>>>> ######### https://listserv.kaliv.uni-karlsruhe.de/warc/bse-l.html> ##########>>